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Objective.To describe the cohort of patients with inflammatory rheumatic diseases (IRD) hospitalized due to SARS-CoV-2 infection in the Ramón y Cajal Hospital, and to determine the increased risk of severe coronavirus disease 2019 (COVID-19) in patients with no IRD.Methods.This is a retrospective single-center observational study of patients with IRD actively monitored in the Department of Rheumatology who were hospitalized due to COVID-19.Results.Forty-one (1.8%) out of 2315 patients admitted due to severe SARS-CoV-2 pneumonia suffered from an IRD. The admission OR for patients with IRD was 1.91 against the general population, and it was considerably higher in patients with Sjögren syndrome, vasculitis, and systemic lupus erythematosus. Twenty-seven patients were receiving treatment for IRD with corticosteroids, 23 with conventional DMARDs, 12 with biologics (7 rituximab [RTX], 4 anti–tumor necrosis factor [anti-TNF], and 1 abatacept), and 1 with Janus kinase inhibitors. Ten deaths were registered among patients with IRD. A higher hospitalization rate and a higher number of deaths were observed in patients treated with RTX (OR 12.9) but not in patients treated with anti-TNF (OR 0.9).Conclusion.Patients with IRD, especially autoimmune diseases and patients treated with RTX, may be at higher risk of severe pneumonia due to SARS-CoV-2 compared to the general population. More studies are needed to analyze this association further in order to help manage these patients during the pandemic.

To evaluate adherence and quality of life to oral antineoplastic treatment in patients with chronic lymphocytic leukemia. To compare adherence and QoL according to treatment subgroups and treatment-line subgroups.We conducted a descriptive prospective study from June to November 2021 in a tertiary care hospital. Patients treated at the Oncology Pharmacy with a diagnosis of chronic lymphocytic leukemia and treatment with oral antineoplastics for at least 6 months before inclusion in the study were included. Adherence was assessed using Morisky's 8 item Medication Adherence Scale and leftover pills counts, considering adherents if their adherence rate was ≥ 90%. Quality of life was assessed with Euro-Qol EQ-5D-3L questionnaire, Functional Assessment of Chronic Illness Therapy - Fatigue scale and QLQ-C30 questionnaire from European Organization for Research and Treatment of Cancer. Two interviews were scheduled: at the time of inclusion and at 3 months. Variable collected: demographic data, clinical data (disease and treatment); and response (scores obtained from questionnaires and adherence rate). The data statistical analysis was carried out with SPSS® 25.0 software.Twenty three patients were included, all of them showed an adherence rate higher than 90%; 20 patients were considered high adherent, and 3 patients medium adherent to treatment according to Morisky's 8 item Medication Adherence Scale. The results of the EQ-5D-3L questionnaire showed that the patients were all of them autonomous in their personal care and daily activities, 69.6% did not have any mobility problems and 78.3% did not have anxiety/depression; 56.5% had some type of pain. Eighteen patients had no fatigue, and 5 had mild/moderate fatigue according to Functional Assessment of Chronic Illness Therapy - Fatigue scale. The results of the EORTC QLQ-C30 questionnaire showed that patients had a high /healthy functional level, a good quality of life and a low level of symptoms. Analysis by treatment subgroups and by treatment-line subgroups did not show statistically significant differences in adherence or quality of life.Patients diagnosed with chronic lymphocytic leukemia and treated with oral antineoplastic therapies showed a high adherence rate and referred a good quality of life.

espanolObjetivo El objetivo de este estudio es describir los hallazgos de una evaluacion odontologica protocolizada en pacientes con sindrome de Sjogren primario (SSp) atendidos en las consultas de reumatologia de la Comunidad de Madrid. Metodos Estudio descriptivo multicentrico en el que se incluyeron pacientes con SSp clasificados segun criterios del consenso Europeo-Americano de 2002. Se recogieron datos demograficos, clinicos y serologicos. Se realizo una exploracion oral, se recogio el flujo salival y el indice de caries CAOD. Los pacientes rellenaron la escala visual analogica para xerostomia, el cuestionario Oral Health Impact Profile-14 y un cuestionario de salud oral. Resultados Se reclutaron 61 pacientes. El 98% fueron mujeres y la edad media fue de 57 anos. El 52,5% presentaban signos orales patologicos (flujo salival no estimulado/sialografia/gammagrafia de glandulas salivales) y el 90,2% antiRo/antiLa+. El 92% de los pacientes referian xerostomia y un 61% hiposialia. Treinta y cinco pacientes presentaron lesiones de la mucosa oral. El indice CAOD fue de 16,97 ± 7,93 y la escala visual analogica para la xerostomia fue de 46,69 ± 14,43. Los resultados del Oral Health Impact Profile-14 fueron de 23,13 ± 14,16. Los pacientes con signos orales patologicos obtuvieron una puntuacion en el Oral Health Impact Profile-14 significativamente mayor (p = 0,03), al igual que los pacientes con afectacion del sistema nervioso periferico (p = 0,001). Conclusiones La prevalencia de xerostomia en esta cohorte de pacientes con SSp fue muy elevada y el 61% de los enfermos presentaron hiposialia. Mas de la mitad de los pacientes sufrieron lesiones orales. La salud oral presento un impacto negativo en la calidad de vida de los pacientes con SSp, siendo mayor en aquellos pacientes con signos orales objetivos patologicos y en los que presentaban afectacion del sistema nervioso periferico. EnglishObjective The aim of this study is to describe the findings of a protocolised odontological evaluation of patients with primary Sjogren's syndrome (pSS) treated in rheumatology units in the Community of Madrid. Methods Multicentric descriptive study in which pSS patients classified according to the American-European consensus of 2002 criteria were included. We collected the demographic, clinical and serological data of each patient. A complete oral examination was performed and salivary flow and the CAOD caries index were collected. The patients completed the visual analogue scale for xerostomia, the Oral Health Impact Profile-14 questionnaire and an oral health questionnaire. Results Sixty-one patients were recruited. Ninety-eight percent were women and the mean age of the patients was 57 years. Pathological oral signs (unstimulated salivary flow/salivary glands sialography/scintigraphy) were present in 52.5% of the patients, anti-Ro/anti-La were positive in 90.2%. Ninety-two percent of the patients reported xerostomia and 61% of the patients suffered from hyposialia. Thirty-five patients presented oral mucosa lesions. CAOD index was 16.97 ± 7.93 and visual analogue scale for xerostomia was 46.69 ± 14.43. The results of the OHIP-14 questionnaire were 23.13 ± 14.16. Patients with pathological oral signs obtained a significantly higher Oral Health Impact Profile-14 score (P = .03). We also found that patients with peripheral nervous system involvement obtained a significantly higher Oral Health Impact Profile-14 score (P = .001) Conclusions The presence of xerostomia in this cohort of pSS patients was high and hyposialia was present in 61% of the patients. Oral lesions appeared in more than half of the subjects. Oral health had a negative impact on the quality of life of patients with pSS, being higher in those with pathological objective oral signs and in those with peripheral nervous system involvement.

Extravasation is a potential complication resulting from parenteral administration of drugs. The purpose of this study was to characterise the physicochemical properties of non-antineoplastic parenterally administered drugs and determine their potential to cause a toxic effect on tissue.A list of drugs administered by intermittent or continuous intravenous (IV) infusion was prepared. A database was also established to collect information from the literature. Each active substance was classified according to its risk to cause tissue damage using the following criteria: (1) High risk: active substances presenting with any of the following characteristics: osmolarity of the IV solution form500 mOsm/L; vasoconstriction; vesication; cellular toxicity; very common, common or uncommon adverse events such as phlebitis, necrosis or pain at the site of administration according to the Summary of Product Characteristics. (2) Moderate risk: active substances where the pH range was3 or11 or where adverse events at the site of administration occurred rarely, very rarely or with unknown frequency. (3) Low risk: active substances where the osmolarity of the IV solution was500 mOsm/L and the pH ranged between 3 and 11. These active substances did not cause vasoconstriction, neither were they classified as vesicant or cytotoxic or presented with adverse events at the site of administration.The risk classification list included 138 active substances, of which 86 were classified as 'high risk', 18 as 'moderate risk' and 34 as 'low risk'.The classification of intravenously administered drugs according to their risk profile is useful to ensure their safe use, as it can be used to implement the necessary safety measures to prevent adverse events.

Introduction Capmatinib is a mesenchymal-epithelial transition (MET) inhibitor authorized for metastatic non-small cell lung cancer (NSCLC) with MET exon 14 skipping mutation treatment in adult patients. Case report We report a case of an elderly female with a diagnosis of metastatic NSCLC with MET exon 14 skipping mutation who developed a severe hepatotoxicity after 7 weeks under treatment with capmatinib. Management & Outcome Capmatinib was immediately discontinued. Hepatotoxicity is included as “warning and precautions” in the product information sheet. The patient was admitted with severe acute hepatitis, secondary hypocoagulability and acute deterioration of renal function. She experienced a rapid worsening with a fatal outcome three days after admission. The causal relationship between capmatinib and the appearance of hepatotoxicity was determined as probable according to Naranjo's modified Karch and Lasagna's imputability algorithm. Discussion The recognition and diagnosis of drug-induced liver injury (DILI) are often difficult and delayed. Molecularly targeted agents require careful assessment of liver function both prior to and during therapy. Capmatinib hepatotoxicity is an infrequent but severe adverse drug reaction (ADR). Prescribing information includes recommendations about liver function monitoring. The main approachment for DILI is the removal of the causative agent. Detection and communication of ADRs to the Pharmacovigilance Systems have special relevance for novel drugs, with little data in real life setting.

To evaluate the properties of a single question to determine patient perception of remission and disease activity; to compare this with existing definitions for remission and low disease activity (LDA).Single-center, cross-sectional study in a tertiary care hospital. Patients with axSpA (fulfilling ASAS criteria) were consecutively included. Both the patient's perception of remission and LDA and that of the physician were evaluated with a single question. Agreement between the patients' perception and other current definitions was tested using the prevalence-adjusted and bias-adjusted kappa (PABAK). The sensitivity (S) and specificity (Sp) of current definitions of disease states were tested against the patient's perception as the gold standard.The study population included 105 axSpA patients (63.8% males; 67.6% with radiographic sacroiliitis). Patients considered themselves to be in remission in 21% of cases and in LDA in 51.4%; physicians considered patients to be in remission in 45.7% of cases and in LDA in 35.2%. The poorest agreement was recorded for the patient's and the physician's perception of remission. The best agreement for patients' perception of remission was for a BASDAI 2 and normal C-reactive protein values. This definition was also the most sensitive (S=72.7%) and specific (Sp=83.1%) when the patient's perception was taken as the gold standard.In a real-life setting, the evaluation of remission by the patient through a single question was the hardest criterion to achieve, although it did prove to be a feasible, valid, and specific way to assess remission.

Introduction One of the missions of the Spanish Society of Rheumatology is to provide the necessary tools for excellence in health care. Currently, there is no reference point to quantify medical actions in this specialty, and this is imperative. Material and method A list of actions was drawn up and a hierarchical classification system was established by developing a complexity index, calculated based on the completion time and difficulty level of each action. Results The results of the Delphi method tended to the consensus opinion within a group (mean σ2 − σ1 = 0 .75–1.43 = −0.68, mean IQR2–IQR1 = 0.8–1.9 = −1.1). The values of the complexity index ranged between 48 and 465 points. Among consultation actions, those reaching the highest scores were the first inpatient visit (366) and visits to the patient's home (369). Among diagnostic techniques, biopsies were prominent, those with the highest score were: bone biopsy (465), sural nerve biopsy (416) and synovial biopsy (380). Ultrasound scan scored 204, capillaroscopy 113 and densitometry 112. Among therapeutic techniques, infiltration/arthrocentesis/articular injection in children reached the highest difficulty (388). The score for ultrasound-guided articular injection was 163. The score for clinical report on disability was 323 and expert report 370. Conclusions A nomenclature of 54 actions in Rheumatology was compiled. Biopsies (bone, sural nerve, synovial), inpatient visits, visits to the patient's home, infiltrations in children, and the preparation of the expert report were identified as the most complex actions. Musculoskeletal ultrasound is twice as complex as subsequent visits, capillaroscopy or bone densitometry.

espanolIntroduccion Una mision de la Sociedad Espanola de Reumatologia es aportar las herramientas necesarias para alcanzar la excelencia asistencial. En la actualidad no existe una referencia que cuantifique la complejidad de los actos medicos de esta especialidad. Material y metodo Se elaboro una relacion de los actos propios del reumatologo y se establecio un sistema de clasificacion jerarquica a partir de la construccion de un indice de complejidad, calculado mediante el tiempo de realizacion y el grado de dificultad de cada acto. Resultados Los resultados del metodo Delphi tendieron a una opinion grupal consensuada (media σ2 - σ1 = 0,75-1,43 = -0,68, media IQR2 - IQR1 = 0,8-1,9 = -1,1). El rango de valores del indice de complejidad oscilo de 48 a 465 puntos. Entre las consultas, las que alcanzaron mayor gradacion fueron la primera visita al paciente hospitalizado (366) y la visita a domicilio (369). Entre las tecnicas diagnosticas, destacaron las biopsias. Las que puntuaron mas alto fueron: biopsia osea (465), de nervio sural (416) y sinovial (380). La ecografia tuvo una puntuacion de 204, la capilaroscopia de 113 y la densitometria de 112. Entre las tecnicas terapeuticas, la maxima dificultad (388), la alcanzo la infiltracion/artrocentesis/ inyeccion articular infantil. La puntuacion de la inyeccion articular con control ecografico fue de 163. El informe clinico de minusvalia, 323 y el informe pericial, 370. Conclusiones Este trabajo ha permitido elaborar un nomenclator de 54 actos en reumatologia donde se identifican como actos mas complejos la realizacion de biopsias (osea, nervio sural, sinovial), la visita a paciente hospitalizado, la visita a domicilio, la infiltracion infantil bajo sedacion y la elaboracion de un informe pericial. La ecografia osteomuscular es considerada el doble de compleja de una visita sucesiva, la capilaroscopia o la densitometria osea. EnglishIntroduction One of the missions of the Spanish Society of Rheumatology is to provide the necessary tools for excellence in health care. Currently, there is no reference point to quantify medical actions in this specialty, and this is imperative. Material and method A list of actions was drawn up and a hierarchical classification system was established by developing a complexity index, calculated based on the completion time and difficulty level of each action. Results The results of the Delphi method tended to the consensus opinion within a group (mean σ2 - σ1 = 0.75-1.43 = -0.68, mean IQR2 - IQR1 = 0.8-1.9 = -1.1). The values of the complexity index ranged between 48 and 465 points. Among consultation actions, those reaching the highest scores were the first inpatient visit (366) and visits to the patient's home (369). Among diagnostic techniques, biopsies were prominent, those with the highest score were: bone biopsy (465), sural nerve biopsy (416) and synovial biopsy (380). Ultrasound scan scored 204, capillaroscopy 113 and densitometry 112. Among therapeutic techniques, infiltration/ arthrocentesis/articular injection in children reached the highest difficulty (388). The score for ultrasound-guided articular injection was 163. The score for clinical report on disability was 323 and expert report 370. Conclusions A nomenclature of 54 actions in Rheumatology was compiled. Biopsies (bone, sural nerve, synovial), inpatient visits, visits to the patient's home, infiltrations in children, and the preparation of the expert report were identified as the most complex actions. Musculoskeletal ultrasound is twice as complex as subsequent visits, capillaroscopy or bone densitometry.

Objective To carry out a systematic review of the literature to analyse the efficacy and safety of treatments available or under investigation for amyloidosis due to mutations in the transthyretin gene (ATTR). Methods A bibliographic search was carried out in the following electronic databases up to September 2017: PubMed, Cochrane Library and EMBASE. The inclusion criteria were: efficacy and/or safety studies conducted in humans, studies that included treatments, including treatments in the research phase, and studies that included 10 or more patients. Results A total of 21 articles were included; 16 were clinical trials, eight of them (50%) phase III trials, and five were observational studies. Of the total number of studies selected, 11 were on tafamidis, four on diflunisal, two on liver transplantation, two on patisiran and two on other therapeutic alternatives. Of the 11 studies related to the drug, the pivotal trial, the results of its two extension studies and an additional post hoc analysis were selected. In addition, two phase III trials were included in specific populations, two phase II studies, one safety study and two observational studies. Regarding the four included studies related to the drug, one was the pivotal trial that gave the indication to diflunisal, another a safety summary of the pivotal trial, and the other two trials were carried out in specific populations, one in a Japanese population and another phase I trial in cardiac amyloidosis in the USA. As far as other alternatives are concerned, of the six studies included in this section, two were related to liver transplantation, two to patisiran and two to different therapeutic alternatives. Conclusions Sufficient evidence has not been found that demonstrates superiority among the available oral alternatives, diflunisal or tafamidis, in the treatment of ATTR. Direct comparisons between both drugs and pharmacoeconomic studies would be necessary to select the most efficient treatment.

Secukinumab is a novel anti-IL17 biologic treatment approved for the treatment of psoriatic arthritis (PsA). The purpose of the present study is to identify factors that can condition the retention rate of this drug in a real-world scenario. Methods: A multicentric retrospective study was conducted based on the registries of consecutive patients diagnosed with PsA who started secukinumab from January 2016 to December 2018. For purposes of Cox-regression analysis, the time spanning from the first administration of secukinumab until its interruption or the end of the follow-up was considered the independent variable. Variables of known relevance and those who demonstrated direct association with the drug retention rate were included in the model. Results: One hundred seventy-six registries were analyzed (average age at diagnosis 44.7 ± 12.1 years old, 114 females). The median retention rate of secukinumab was 636 days (95% confidence interval [CI] 542.4-729.5). Presence of peripheral arthritis (hazard ratio 0.424 [95% CI 0.213-0.847, P = .015]) and a time of evolution 6 years (hazard ratio 0.468 [95% CI 0.225-0.975, P = .043]) were the 2 variables that showed a significant influence on the drug retention rate. According to our results, patients who exhibit peripheral arthritis and those with a higher evolution time will have more probabilities of a larger secukinumab retention rate.

Background In immune-mediated inflammatory rheumatic diseases (IMIRD), there are differences between cis-men and cis-women in epidemiology, clinical feature, therapeutic approach, treatment response, and prognosis. In transgender individuals, information concerning IMIRD is not substantial. The assessment of information concerning rheumatic diseases in transgenders is crucial because transgenders may undergo treatments with potential impacts on IMIRD. We aim to collect and discuss current knowledge on IMIRD in transgender individuals, determine the coverage of the literature, identify the knowledge gaps, and highlight opportunities for future research. Methods We did a scoping review of publications collected through a systematic search of transgender patients with any IMIRD. Data sources were Medline, Embase, and Web of Knowledge. Synthesis of results and qualitative review of data information was collected in tables. A semi-quantification of the quality of the articles reporting clinical studies was performed. Results There were 11 transwoman, and 3 transmen cases of systemic lupus erythematosus (5 cases), skin lupus erythematosus (2), systemic sclerosis (4), anti-synthetase syndrome (1), rheumatoid arthritis (1) and ankylosing spondylitis (1). Eleven were de novo cases and three had prior history of IMIRD and developed a comorbidity after starting hormone replacement therapy. The clinical expression of the disease was variable. Two transwomen and one transman developed thrombotic events. The lupus skin lesions in one transman improved following testosterone treatment. No clinical studies were identified. Quality of publications was disparate. Conclusion Although the number of cases is small, most cases of IMIRD occur in transwomen. The absence of solid data warrants caution in establishing recommendations regarding hormone replacement therapy in transgenders with IMIRD. There is an essential need for the consideration of cisgender and transgender particularities in future research on IMIRD

SummaryAggressive behavior is an ancient and conserved trait considered habitual and essential for most animals in order to eat, protect themselves from predators and also to compete for mating and defend their territories. Genetic factors have shown to play an important role in the development of aggression both in animals and humans, displaying moderate to high heritability estimates. However, although such types of conducts have been studied in different animal models, the molecular architecture of aggressiveness remains poorly understood. This study compared gene expression profiles of 16 prefrontal cortex (PFC) samples from aggressive and non-aggressive cattle breeds: Lidia, selected for agonistic responses, and Wagyu, specialized on meat production and selected for tameness. RNA sequencing was used to identify 918 up and 278 down-regulated differentiated expressed genes (DEG). The functional interpretation of the up-regulated genes in the aggressive cohort revealed enrichment of pathways such as the Alzheimer disease-presenilin, integrins or the ERK/MAPK signaling cascade, all implicated in the development of abnormal aggressive behaviors and neurophysiological disorders. Moreover, gonadotropins, leading to testosterone release, are also up-regulated as natural mechanisms enhancing aggression. Concomitantly, heterotrimeric G-protein pathways, associated with low reactivity mental states, and the GAD2 gene, a repressor of agonistic reactions at PFC, are down-regulated, guaranteeing the development of the adequate responses required by the aggressive Lidia cattle. We also identified six upstream regulators, whose functional activity fits with the etiology of abnormal behavioral responses associated with aggression. These results provide valuable insights into the complex architecture that underlie naturally developed agonistic behaviors.

Background and objective. Inotuzumab ozogamicin (InO) was approved for patients (pts) with relapsed/refractory (R/R) CD22-positive acute lymphoblastic leukemia (ALL) based on the results of INO-VATE trial (Kantarjian et al, 2016). There are scarce studies evaluating the results of InO therapy in real life in similar pts as those from the INO-VATE trial. Our objective was to analyze the outcomes of pts included in the compassionate program of InO in Spain (June 2013-April 2018) before definitive approval. Patients and Methods. Inclusion criteria were age >18 yrs., CD22+ ALL, R/R resistant to ≥2 previous lines, Ph+ ALL resistant/intolerant to TKI, ECOG ≤2 or >2 if due to ALL, Bilirubin Results. 34 pts were included in the trial, 21 males, median age 43 yrs (range 19-73), ECOG 50% 15/33 (45%). 25/34 (73%) of pts received >2 previous lines of therapy and 20 (59%) were previously transplanted. The duration of first CR remission before InO was The median number of InO cycles was 2 (1-6). One pt withdrew the study before evaluation, 5 (15%) dead during therapy and 21 (64%) achieved CR/CRi. Ten pts (29%) were transplanted. With a median follow-up for alive patients after InO start of 26 months, the medians (95%CI) of DR, PFS and OS were 4.7 months (2.4-7.0), 3.5 (2.0-5.0) and 4.0 months (1.9-6.1), respectively. CR duration, PFS and OS were significantly shorter in refractory ALL (Figure 1A), pts with first CR (CR1) duration The most frequent adverse events were hepatic (24%), infectious (18%), hematologic (15%) and gastrointestinal (9%). 3/10 transplanted patients showed grade 3-4 VOD/SOS. Grade 5 toxic events were hepatic (n=2), infection (n=2) and hemorrhage (n=1). Conclusion. The results in this series of compassionate use of InO for R/R ALL before approval for clinical use were slightly inferior to that of the INO-VATE trial. However, patients form this series had poorer risk factors than those included in that trial. The frequency and type of AE were similar to that of observed in the INO-VATE trial. Supported in part by grant 2017 SGR288 (GRC) Generalitat de Catalunya and "La Caixa" Foundation. Figure 1. Overall survival according to ALL status at inotuzumab start (A) and to duration of first complete remission (B) Figure 1 Figure 1. Disclosures Ribera: AMGEN: Consultancy, Research Funding, Speakers Bureau; SHIRE: Consultancy, Speakers Bureau; ARIAD: Consultancy, Research Funding, Speakers Bureau; TAKEDA: Consultancy, Research Funding, Speakers Bureau; NOVARTIS: Consultancy, Speakers Bureau; Pfizer: Consultancy, Research Funding, Speakers Bureau. Hernández-Rivas: Celgene/BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Pfizer: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria, Membership on an entity's Board of Directors or advisory committees.

Background Prognostic factors of poor outcome in patients with hematological malignancies and COVID-19 are poorly defined. Patients and methods This was a Spanish transplant group and cell therapy (GETH) multicenter retrospective observational study, which included a large cohort of blood cancer patients with laboratory-confirmed SARS-CoV-2 infection through PCR assays from March 1st 2020 to May 15th 2020. Results We included 367 pediatric and adult patients with hematological malignancies, including recipients of autologous (ASCT) (n = 58) or allogeneic stem cell transplantation (allo-SCT) (n = 65) from 41 hospitals in Spain. Median age of patients was 64 years (range 1–93.8). Recipients of ASCT and allo-SCT showed lower mortality rates (17% and 18%, respectively) compared to non-SCT patients (31%) (p = 0.02). Prognostic factors identified for day 45 overall mortality (OM) by logistic regression multivariate analysis included age > 70 years [odds ratio (OR) 2.1, 95% confidence interval (CI) 1.2–3.8, p = 0.011]; uncontrolled hematological malignancy (OR 2.9, 95% CI 1.6–5.2, p 20 mg/dL (OR 3.3, 95% CI 1.7–6.4, p

Background Secukinumab, an inhibitor of IL-17, is a new option for the treatment of Psoriatic Arthritis (PsA) which has showed efficacy in clinical trials. However, real-world data of its use is still scarce. Objectives This study aims to analyze the experience of using secukinumab for PsA in four tertiary hospitals. Methods Multicentric observational, longitudinal, retrospective study conducted in 4 tertiary hospitals of the Madrid region. Patients with clinical diagnosis of PsA and having received at least one dose of secukinumab were included. Medical records were reviewed to collect demographic and clinical data (body mass index, BMI, risk factors cardiovascular disease, cancer, HBV/HCV infection), features of PsA (extra-articular involvement, radiological damage), previous therapies, assessment of the disease and response data at 6 months of secukinumab (joint counts, CRP, DAPSA, reasons for discontinuation, adverse events). Descriptive statistic analysis using mean and standard deviation was performed. Results 177 patients, of which 115 female (65%) were included. Mean age was 53 y.o.(SD 15) and average duration of the disease was 9 years (SD 7). 169 patients (95%) had peripheral disease (34% joint erosions), 84 (47%) had axial disease (68 with radiological damage), 148 (84%) had psoriasis, 61 (34%) showed dactilitis and 111 (63%) had enthesitis. Average BMI was 28.4 (SD 6), with an obesity rate of 37% (52 pt). Observed comorbidities were hypertension (22 pt, 12%), diabetes mellitus (22 pt, 12%), dyslipidemia (58 pt, 33%), active smoking (55 pt, 31%) and others: 20 patients had HBV infection, 5 had HCV infection and 12% had malignancy. Regarding previous treatments, 90% had received cDMARDs, particularly methotrexate (77%) and 119 (67%) had been exposed to at least one bDMARD (32% to one, 25% to two, 20% to three and 21% to four or more). 69% were on 150 mg dose and 31% on 300 mg dose. At baseline, average tender joint count was 7 (SD 8), swollen joint count was 4 (SD 4), CRP 7 mg/l and DAPSA 26. At 6 months of secukinumab therapy, tender joint count had decreased to 5 (SD 8), swollen joint count 2 (SD 3), CRP 5 mg/l and DAPSA 17. Thirty-eight (47%) of the patients with the data available (80) had DAPSA ≤14 (low activity) and 9 DAPSA ≤4 (remission). In naive to biologic patients, DAPSA varied from 27 in the baseline visit to 16 at 6 months, and in the other group of patients with biologic experience, DAPSA varied from 24 to 17 at 6 months (if they had been exposed to 3 or more biologics, DAPSA from 18 to 10 at 6 months). Average drug survival time was 20 months (1-34). 79 patients (44.6%) withdrawn therapy, due to primary ineffectiveness (40), secondary ineffectiveness (26), adverse events (9) and other reasons (3). Adverse events do not differ from those reported in clinical trials. Conclusion Secukinumab in real-world setting had been indicated in a population of PsA with a high percentage of axial involvement (47%), with an important previous exposure to biologics, and with higher comorbidity than that reported in clinical trials. Response data were favorable, 47% of patients achieved remission or low activity by DAPSA, and similar profile of adverse events to the one reported in the clinical trials was observed. References [1] Mease PJ, McInnes IB, Kirkham B, Kavanaugh A, Rahman P, van der Heijde D, et al. for the FUTURE 1 Study Group* Secukinumab Inhibition of Interleukin-17A in Patients with Psoriatic Arthritis. N Engl J Med2015;373:1329-39 Disclosure of Interests MARIA MARTIN LOPEZ: None declared, Marta Valero: None declared, Valentina Emperiale: None declared, Carolina Merino Argumanez: None declared, Javier Bachiller-Corral: None declared, Jose Campos Esteban: None declared, Ana Perez Gomez: None declared, Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, AbbVie, and Janssen

Background Secukinumab is a newly introduced biologic therapy against IL-17 which has been approved for Psoriatic arthritis and has showed efficacy in clinical trials, but real world data is still lacking. Objectives This study aims to analize secukinumab drug survival for psoriatic arthritis in a real world environment. Methods Multicentric observational, longitudinal retrospective study conducted at 4 tertiary hospitals in Madrid region. Patients with clinical diagnosis of psoriatic arthritis which had received at least one dose of secukinumab between january 2016 and october 2018 were included. With follow up period till December 31, 2018. Medical records were reviewed to collect data about psoriatic arthritis involvement, comorbidities, previos DMARD and bDMARD therapies and its reasons for discontinuation, duration of secukinumab therapy, reasons for discontinuating secukinumab therapy and adverse events. Statistical analysis was performed including bivariate analisys (considering withdrawal of drug during study period or not) and survival analysis with Kaplan-Meier and Cox regression. Results 177 patients that initiated secukinumab therapy in the recruitment period were included. 123 pacients were on 150 mg dose and 54 on 300 mg dose. Average follow up period was 13 months (SD 8,28; range 1-34) in the bivariate analysis, a higher proportion of biologic-naive patients was found among the group without secukinumab withdrawal during the study (40% vs 23%, p 0.07). No other differences were observed between both groups regarding demographic characteristics nor comorbidities (tobacco exposure, hypertension, diabetes mellitus, dyslipidemia, ischemic heart disease or malignancy). Median survival time for secukinumab in the Kaplan-Meier analysis was 21.2 months (IC 95%: 14.3 - 22.2), with an average of 19,9 months. One event was censored due to lost to follow-up. Secukinumab treatment was withdrawn in 79 patients (44.6%). Reasons for discontinuation were lack of effectiveness (37%), either primary (40 patients) or secondary (26 patients), adverse events (9 patients, 0,5%), elective surgery in one patient and latex allergy in other. The only variable associated to higher drug survival in Cox regression analysis was biologic-naive status. No differences in drug survival were found in all other variables studied (gender, age, secukinumab dosage, illness duration, clinical features, tobacco exposure and rest of comorbidities). Conclusion As secukinumab was marketed in 2016, real-word setting studies lack long term data. The mean follow up period in our study was 13 months. Most of withdrawals were to lack of effectiveness. Median survival was 21.2 months, significatively higher in biologic-naive patients. No other differences were found in all other variables studied. Probably real-world data differ from those of clinical trials. Disclosure of interests Marta Valero: None declared, Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, abbVie, and Janssen, Maria Martin: None declared, Jose Campos Esteban: None declared, Carolina Merino argumanez: None declared, Valentina Emperiale: None declared, ana Perez Gomez: None declared, Javier Bachiller-Corral: None declared

Background IL-17 inhibition has been proved effective in patients with ankylosing spondylitis (AS) in clinical trials and it has been added to the most recent national and international treatment guidelines. However, real-world data of its use is still scarce. Objectives This study aims to describe the axial spondyloartrhtitis (AxSpA) population receiving secukinumab therapy under non-experimental conditions. Methods Multicentric observational, retrospective, longitudinal study conducted in 4 tertiary hospitals of the Madrid region. Patients over 18 y.o. With clinical diagnosis of axSpa and having received at least one dose of secukinumab were included. Medical records were reviewed to collect demographic and clinical data related to axSpA, its features and treatment. Descriptive statstic analysis with measures of central tendency and measures of variability was performed. Results 143 patients, of which 68 women (47.6%) were included. Mean age was 50.6 y.o.(26-74) and average duration of the disease was 12.4 years (1-54). 105 aS patients (73.4%) and 38 non radiographic axSpA patients (26.6%). 85 of them were HLAB27 positive (62%) Observed comorbidities were hypertension (37pt, 25.8%), diabetes mellitus (12pt, 8.4%), dyslipidemia (43 pt,30.1%) and active smoking (41pt,28.7%) average BMI was 27.1 (15.8-43.4), with an obesity rate of 27.6% (29pt). Other comorbidities highlighted were ischemic heart disease (10pt, 7%), HBV infection (10pt, 7.5%), malignancy (14pt, 9.8%) and renal transplantation (2 pt, 1.4%). No HVC nor HIV infections were detected. In terms of axial disease, 97 (65.7%) patients showed grade 2 or higher sacroilitis. In sacroiliac joint MRI studies 35 patients (42.7%) had acute and chronic lesions, 13 (15.9%) had acute lesions, 17 (20.7%) had chronic lesions and 17 (20.7%) were normal. Syndesmophytes were present in 49 patients (34.5%) and hip arthropathy in 15 patients (11.4%). In addition, peripheral disease was present: 77 patients (53.8%) had arthritis, 80 patients (55.9%) had enthesitis and 9 patients (6.3%) showed dactilitis. Joint erosions in hand and feet X-rays were reported in 6 patients (6.8%). Extraarticular SpA features found were inflammatory bowel disease in 3 patients (2.1%), uveitis in 17 pts (11.9%) and psoriasis in12 pts (8.4%). 2 patients (1.4%) presented secondary amiloidosis. Regarding previous treatments, 97.2% had used NSAIDs and 38.5% glucocorticoids. 100 patients (84.1%) had received DMARDs, particularly sulfasalazine (84 pts, 58.7%). 38 patients were naive to biologic therapy (26.6%); 36 (34.3%) had been exposed to one bDMARD, 39 (37.1%) to two and 30 (28.6%) to three or more. Besides all TNF inhibitors, 13 treatments with abatacept, tocilizumab, rituximab or ustekinumab were recorded. Average baseline BASDAI was 6.54 (0.4-12.8), and 5.01 (0-10) after 6 mo SCK therapy. Baseline BASFI was 5.8 (0.4-10) and 4.8 (0-9.6) at 6 months. Adverse events did not differ from those reported in clinical trials. Conclusion Population receiving SCK in real-world setting is different to clinical trials: more complex patientes, with higher disease activity, higher use of previous bDMARDs and higher comorbidity, particularly cardiovascular risk, infections or malignancy that could preclude the use of other biologic therapies. A relevant percentage of off-label prescription in nrAxSpA is reported. Further studys are needed to characterize this population and its clinical response and tolerability to SCK therapy. Disclosure of interests Valentina Emperiale: None declared, Jose Campos Esteban: None declared, Carolina Merino argumanez: None declared, Javier Bachiller-Corral: None declared, Marta Valero: None declared, Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, abbVie, and Janssen, Maria Martin: None declared, ana Perez Gomez: None declared

Background:Current recommendations for axial Spondyloarthritis (axSpA) include the treat-to-target concept and suggest that the ideal target should be remission or low disease activity (LDA). Also, the importance of a shared decision is highlighted. Unfortunately, the definition of remission is not consensual, and most of the definitions are difficult to evaluate in clinical practiceObjectives:To propose an evaluation of remission by a single question to the patient, by comparing it to the different available definitions. To analyze the metric properties of the current definitions against patient’s perceptionMethods:One-center cross-sectional study in a tertiary care hospital including consecutive patients with a diagnosis of axSpA (and fulfilling the ASAS criteria) were included between February to November 2019. Patient’s perception of remission and LDA was evaluated by a single question. Physician’s perception of remission and LDA was assessed identically. The level of agreement between patients’ perception and the other available definitions was tested by the Prevalence and Bias adjusted Kappa (PABAK). The metric properties Sensitivity (S) and Specificity (Sp) of the available definitions (BASDAI cut-offs, ASDAS disease states, ASAS criteria for partial remission and patient acceptable symptom state), were tested against the patients perspective, as the gold standard.Results:A total of 105 axSpA patients were included. 63,8% were males and 67,6% had radiographic sacroiliitis (Table 1). 21% and 72% of them considered themselves in remission and LDA, respectively. Physician’s perception was 45.7% and 81% for remission and LDA, respectively. The prevalence of the different definitions are shown in Figure 1. The best agreement for patients’s perception of remission was found with a BASDAI Table 1.Characteristics of 105 patients with axSpAAll (N:105)Patients in self-defined REM (N:22)Patients in self-defined LDA (N:54)Patients No REM no LDA (N:29)Male, n (%)67 (63,8)18 (81,8)34 (63)15 (51,7)r-axSpA, n (%)71 (67,6)17 (77,3)33 (61,1)21 (72,4)Mean age, years (SD)49 (13)51(15)47 (13)50 (11)Mean AxSpA duration, years (SD)12,2 (13)17,1 (16,2)11,2 (11,7)10,3 (12,3)HLA- B27+, n (%)Data from 10472 (69,2)17/22 (77,3)33/54 (61,1)22/28 (78,6)Periferic arthritis, n (%)34 (32,4)7 (31,8)17 (31,5)10 (45,4)Uveitis, n (%)22 (21)6 (27,3)10 (18,5)6 (20,7)Biological treatment, n (%)43 (41)14 (63,6)19 (35,1)10 (34,5)CRP, mean (SD)3,61 (5,36)2,31 (2,17)2,84 (3,87)6,04 (8,14)ASDAS, mean (SD)1,78 (1,08)0,98 (0,71)1,63 (0,89)2,68 (1,03)BASDAI, mean (SD)3,35 (2,32)1,39 (1,30)3,13 (1,84)5,26 (2,33)BASFI, mean (SD)2,81 (2,45)1,24 (1,37)2,57 (2,00)4,43 (2,92)Table 1.REM: Remission; LDA: Low Disease Activity; SD: Standard Desviation; CRP: C-Reactive Protein IBD: Inflammatory Bowel Disease.Table 2.Agreement between different definitions of remissionASDAS BASDAINormal CRPPGA ≤1PhysicianREMPatientREMASAS PR0.53 (0.58)0.59 (0.68)0.76 (0.83)0.22 (0.26)0.39 (0.56)ASDAS 0.64 (0.68)0.50 (0.56)0.44 (0.45)0.28 (0.37)BASDAI Normal CRP0.60 (0.69)0.25 (0.28)0.50 (0.62)PGA ≤10.20 (0.24)0.42 (0.62)Physician REM0.20 (0.24)Agreement is presented as Cohen’s Kappa (PABAK: prevalence and bias adjusted kappa).Patient and Physician remission (REM) are based on the single question; ASAS PR:ASAS partial remission; PGA: Patient global assessment.Conclusion:In this real-life population, the evaluation of remission by the patient through a single question was shown to be feasible and to present an acceptable agreement with other definitions.References:[1]Gorlier C, et al. Ann Rheum Dis 2019;78(2):201-8.Fig. 1.REM/LDA: remission/ low disease activity self-defined patient or physician through a simple question. ASDAS Acknowledgments:To Ansgar Seyfferth and Alfonso Muriel. To Carlos Sanchez-Piedra, Fernando Alonso and Mercedes Guerra from Sociedad Española de Reumatología, Research Unit.Disclosure of Interests:Marta Valero Grant/research support from: Novartis, Pfizer, Abvie, Speakers bureau: Novartis, Celgene, Javier Bachiller-Corral: None declared, Boris Anthony Blanco Cáceres: None declared, M. Ángeles Blázquez: None declared, Consuelo Díaz-Miguel: None declared, Maria Jesus Garcia Villanueva: None declared, Carmen Larena: None declared, Jose Luis Morell Hita: None declared, Carlos De la Puente Bujidos: None declared, Ana Rodriguez-García: None declared, Mónica Vázquez Díaz: None declared, Anna Moltó Grant/research support from: Pfizer, UCB, Consultant of: Abbvie, BMS, MSD, Novartis, Pfizer, UCB

The aim of this study is to describe the findings of a protocolised odontological evaluation of patients with primary Sjögren's syndrome (pSS) treated in rheumatology units in the Community of Madrid.Multicentric descriptive study in which pSS patients classified according to the American-European consensus of 2002 criteria were included. We collected the demographic, clinical and serological data of each patient. A complete oral examination was performed and salivary flow and the CAOD caries index were collected. The patients completed the visual analogue scale for xerostomia, the Oral Health Impact Profile-14 questionnaire and an oral health questionnaire.Sixty-one patients were recruited. Ninety-eight percent were women and the mean age of the patients was 57 years. Pathological oral signs (unstimulated salivary flow/salivary glands sialography/scintigraphy) were present in 52.5% of the patients, anti-Ro/anti-La were positive in 90.2%. Ninety-two percent of the patients reported xerostomia and 61% of the patients suffered from hyposialia. Thirty-five patients presented oral mucosa lesions. CAOD index was 16.97±7.93 and visual analogue scale for xerostomia was 46.69±14.43. The results of the OHIP-14 questionnaire were 23.13±14.16. Patients with pathological oral signs obtained a significantly higher Oral Health Impact Profile-14 score (P=.03). We also found that patients with peripheral nervous system involvement obtained a significantly higher Oral Health Impact Profile-14 score (P=.001) CONCLUSIONS: The presence of xerostomia in this cohort of pSS patients was high and hyposialia was present in 61% of the patients. Oral lesions appeared in more than half of the subjects. Oral health had a negative impact on the quality of life of patients with pSS, being higher in those with pathological objective oral signs and in those with peripheral nervous system involvement.

Resumen El linfoma es la forma mas prevalente de neoplasia hematologica, clasificandose en dos grandes grupos, Hodgkin y no Hodgkin. El linfoma de celulas del manto (LCM) es un subtipo de linfoma no Hodgkin tipo B, de caracter agresivo y que tiene su origen en celulas de la zona periferica del centro germinal o en la zona del manto del foliculo linfoide. El LCM es uno de los linfomas menos frecuentes, suponiendo cerca del 7% de los casos de linfoma no Hodgkin en los EE. UU. y Europa, con una incidencia aproximada de 4 a 8 casos por millon de personas y ano. En torno a tres cuartas partes de los pacientes son varones caucasianos, reduciendose a la mitad la frecuencia en la raza negra. La edad media en el diagnostico es de 68 anos, desarrollando adenopatias en el 75% de los casos y produciendose diseminacion extranodal en el 25% restante (bazo, medula osea, sangre, tracto gastrointestinal, mama, pleura y anejos oculares). El laboratorio es un pilar clave en el diagnostico de LCM establecido mediante el estudio histologico, inmunofenotipico y molecular. El 70% de los pacientes que se diagnostican presentan la enfermedad en un estadio avanzado. Ello conlleva frecuentemente la extension gastrointestinal y a medula osea. Aunque los linfomas afectan principalmente a ganglios linfaticos y tejidos linfoides, en algunos casos invaden la medula osea y sangre periferica. Este proceso recibe el nombre de leucemizacion y ocurre en el 35% de los casos. Ante una cifra de leucocitos elevada en un paciente diagnosticado de LCM, el cometido del laboratorio sera observar el frotis de sangre periferica en busca de linfocitos de aspecto centrocitico. Con los tratamientos convencionales, la supervivencia media era de 3 anos, cifra que ha aumentado hasta los 7 anos para los pacientes que reciben los nuevos tratamientos. El pronostico de LCM leucemizado es inferior al de las formas ganglionares sin expresion periferica.

Objective To design a novel ex vivo acquisition technique to establish a common framework to validate different segmentation techniques for oncological PET images. To evaluate several automatic segmentation algorithms on this set of images. Material and methods In 15 patients with cancer, ex vivo PET studies of surgical specimens removed during surgery were performed after injection of 18 F-FDG. Images were acquired in two scanners: a clinical PET/CT and a high-resolution PET scanner. Real tumor volume was determined in each patient, and a reference image was generated for segmentation of each tumor. Images were segmented with 12 automatic algorithms and with a standard method for PET (relative threshold at 42%) and results were evaluated by quantitative parameters. Results It has been possible to demonstrate by segmentation of PET images of surgical specimens that on high resolution PET images, 8 out of 12 evaluated segmentation techniques outperformed the standard method, whose value is 42%. However, none of the algorithms outperformed the standard method when applied on images from the clinical PET/CT. Due to the great interest of this set of PET images, all studies have been published on the Internet in order to provide a common framework for validation and comparison of different segmentation techniques. Conclusions We have proposed a novel technique to validate segmentation techniques for oncological PET images, acquiring ex vivo PET studies of surgical specimens. We have demonstrated the usefulness of this set of PET images by evaluating several automatic segmentation algorithms.